Circumcision

Chullin 47b ~ Red Babies, Green Babies, and other Neonatal Colors

חולין מז, ב

Green Baby.jpg

רבי נתן אומר פעם אחת הלכתי לכרכי הים באתה אשה אחת לפני שמלה בנה ראשון ומת שני ומת שלישי הביאתו לפני ראיתיו שהיה אדום אמרתי לה בתי המתיני לו עד שיבלע בו דמו המתינה לו ומלה אותו וחיה והיו קורין אותו נתן הבבלי על שמי

ושוב פעם אחת הלכתי למדינת קפוטקיא באתה אשה לפני שמלה בנה ראשון ומת שני ומת שלישי הביאתו לפני ראיתיו שהיה ירוק הצצתי בו ולא היה בו דם ברית אמרתי לה בתי המתיני לו עד שיפול בו דמו המתינה לו ומלה אותו וחיה והיו קורין אותו נתן הבבלי על שמי

 Rabbi Natan says: Once I went to the cities overseas, where one woman came before me who circumcised her first son and he died, and she circumcised her second son and he died, and out of concern that circumcising her third son might cause him to die as well, she brought him before me. I saw that he was red, so I said to her: My daughter, wait for him until his blood is absorbed into him. She waited for him until his blood was absorbed into him and then circumcised him, and he survived. And they would call him Natan the Babylonian after my name. 

Rabbi Natan further related: And on another occasion I went to the state of Cappadocia, and a woman came before me who circumcised her first son and he died, and she circumcised her second son and he died. Out of concern that circumcising her thirds on might cause him to die as well, she brought him before me. I saw that he was green. I looked at him and saw that he did not have the blood of circumcision in him, [i.e., he had a deficiency of blood such that no blood would emerge from the circumcision]. I said to her: My daughter, wait until his blood enters him. She waited for his blood to increase and then circumcised him, and he survived. And they would call his name Natan the Babylonian after my name.

Hemophilia A

Normal father carrier mother.jpg

We have had several occasions in the past to review cases similar of baby boys and the dangers of circumcision, most recently when we studied Chullin 4a and its relation to Hemophilia A. But this is the first time there is a discussion of skin color. Rabbi Natan describes a red looking baby boy and a green looking baby boy. Since in each case there were siblings who had died under similar conditions, it is reasonable to assume that these cases too were caused by hemophilia A. Rabbi Natan’s babies survived because they did not have two copies of the gene for hemophilia. Instead they carried only one copy of the gene which is the cause of this disease. It is a mutation in the F8 gene,which controls the manufacture of Factor VIII, a key component of the clotting cycle. You can see this in the diagram (showing a normal father and carrier mother.) Only half the boys will (on average) become hemophiliacs.

However, there are alternative suggestions, which do not ascribe these colors to hemophilia alone. Let’s start with…

The Red baby boy

In his classic work התלמוד וחכמת הרפואה -The Talmud and Medicine (Berlin 1928, p231-2), I.L Katzenelsohn claimed that the red baby disease is erythema neonatorum. His family also carried hemophilia (because his two brothers had bled to death after their circumcisions), but this third baby was also red. Erythema (toxicum) neonatorum is a common finding in newborns and is thought to be due to an immune reaction (though to what remains unknown). It is a benign condition, and needs no treatment. Rabbi Natan believes that a presumption of hemophilia can only be made once it has been seen three times. This had not yet happened (two previous brothers had died, not three) and so he ordered that the brit can go ahead once the redness cleared up. Luckily, the boy was only a carrier of hemophilia (he was heterozygous for the F8 gene), and so he survived the circumcision.

Others are not so sure what this redness indicated. “I have no idea to what the rabbis are referring to" wrote Dr. Abraham Abraham (that’s his real name, not a typo,) in his work on medical halacha נשמת אברהם, The Soul of Avraham (יורה דעה 263:3). He was a Professor of Medicine at Hebrew University and Hadassah Medical School, so if he is stumped I guess we all are. So now let’s turn to…

The Green Baby Boy

This one is really hard to figure out, because there isn’t agreement on precisely what is meant by the description that the baby is ירוק, green. Rashi describes it as “the color of grass” which is - at least in the summer - a nice green color. Tosafot, being Toasafot, disagrees, and suggests that it is a sky blue color. And even more colors appear in later halachic works.

Katzenelsohn believed the “green” color is more of a yellow one, which is common in neonatal jaundice. (“Where is the best place to detect neonatal jaundice?” I was asked as a medical student. “Um, the sclera?” I offered in reply. “Nope said the doctor, happily pimping me, “in the parking lot, - where the sun provides the best illumination.” But I digress). Neonatal jaundice is an almost universal finding in newborns, and results from a breakdown of the fetal hemoglobin that is no-longer needed now that the baby is ex-utero. Julius Preuss (Biblical-Talmudic Medicine, Jason Aronson 1978 p167) believes the green color does not mean green, but instead it means pale. In this reading the baby was anemic, meaning that it lacked hemoglobin.

And what would the green-is-really blue suggestion of Tosafot indicate? Well, blue skin is called cyanosis, and results from too-little oxygen reaching the tissues. The feet and toes of newborns are often blue for a couple of days, as they develop their own oxygen carrying capacity (having spent nine happy months relying on mom’s). Some of the things that cause peripheral cyanosis include the cold (that’s when your arteries no-longer want to shunt warm blood to your freezing fingers), heart failure (the heart is too tired to pump oxygenated blood to the peripheries) or chronic lung disease (the lungs just can’t get enough oxygen through them and into the bloodstream). Central cyanosis is usually much more serious. It can be caused by infections like pneumonia (and did I mention that it was a defining feature of impending death during the Great Influenza Pandemic of 1918?) In the context of a newborn, it may indicate congenital heart disease. Some forms of congenital heart disease are fatal, but today there are a number of operations (or sometimes several) that can save the neonate’s life.

The Overarching Principle of Brit Milah

Whatever the color of the little baby boy or the number of brothers who may have died as a result of circumcision, Jewish law has evolved from these descriptions in the Talmud to a position that is clear and absolute.

שולחן ערוך יורה דעה רס’ג, א

באלו הדברים שאין מלין ולד שיש בו חשש חולי דסכנת נפשות דוחה את הכל שאפשר לו למול לאחר זמן ואי אפשר להחזיר נפש אחת מישראל לעולם

In all of these conditions in which we do not circumcise the baby because of the risk of a fatal outcome, the circumcision is postponed to a later date [when the child is now healthy]. For it is possible to postpone the day of circumcision, but it is impossible ever to replace the soul of a Jewish baby.

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Chullin 4b~ Circumcision, Shechita, and Hemophilia A

In tomorrow’s page of Talmud, we are deeply engrossed in the question of who is qualified to be a shochet, one who slaughters food in accordance with Jewish law. A baraita (a set of oral teachings that never quite made it into the Mishnah) is quoted that teaches that an uncircumcised man may be a shochet. Then the Talmud asks:

חולין ד, ב

ה"ד אילימא מתו אחיו מחמת מילה האי ישראל מעליא הוא אלא פשיטא מומר לערלות וקא סבר מומר לדבר אחד לא הוי מומר לכל התורה כולה

This uncircumcised man, what are the circumstances? If we say that he is an uncircumcised man whose brothers died due to circumcision and the concern is that he might suffer a similar fate, clearly he may slaughter, as he is a full-fledged Jew and not a transgressor at all. Rather, it is obvious that he is a transgressor with regard to remaining uncircumcised, as he refuses to be circumcised, and the tanna holds that he may nevertheless slaughter an animal since a transgressor concerning one matter is not a transgressor concerning the entire Torah.

The Talmud here is referring to the sad case in which a mother loses her sons because they bleed to death following circumcision. In this encore presentation, let’s revisit the topic, which we first met way back in Yevamot 64a.

יבמות סד, א

תניא מלה הראשון ומת שני ומת שלישי לא תמול דברי רבי רבן שמעון בן גמליאל אומר שלישי תמול רביעי לא תמול... א"ר יוחנן מעשה בארבע אחיות בצפורי שמלה ראשונה ומת שניה ומת שלישית ומת רביעית באת לפני רבן שמעון בן גמליאל אמר לה אל תמולי

It was taught: If she circumcised her first son and he died, and her second son and he too died, she should not circumcise her third son, so taught Rebbi.  Rabbi Shimon ben Gamliel stated that she should indeed circumcise her third child, but [if he died] she must not circumcise her fourth...Rabbi Yochanan said that there was once a case in Zippori in which four sisters had sons:  The first sister circumcised her son and he died, the second sister circumcised her son and he died, the third sister circumcised her son and he died, and the forth sister came to Rabbi Shimon ben Gamliel and he told her "you must not circumcise your son" (Yevamot 64a).

The Talmud here is describing a disease that is affected through the maternal line (hence the four sisters - all of whom seem to pass this disease on to their male children). The disease is X-linked Hemophilia A; the term X-linked indicates that the faulty gene is carried on the X chromosome, which is men is always inherited from the mother. Hemophilia A is an X-linked recessive genetic disease, first described by the American physician John Conrad Otto, who in 1803 described a bleeding disorder that ran in families and mostly affected the men. John Hay from Massachusetts published an account of a "remarkable hemorrhagic disposition" in the New England Journal of Medicine in 1813.

Hay, John. Account of a Remarkable Haemorrhagic Disposition, Existing in Many Individuals of the Same Family.  New England Journal of Medicine  1813:2;3;221-225.

Hay, John. Account of a Remarkable Haemorrhagic Disposition, Existing in Many Individuals of the Same Family. New England Journal of Medicine 1813:2;3;221-225.

 If the mother is a carrier  - as were each of the four sisters in Zippori - then she has a one in four chance of passing on the disease to a child, and that affected child will always be a son:

Courtesy  NHLBI

Courtesy NHLBI

The rabbis argued over a technical point - that is, how many cases of bleeding are needed to establish a pattern. According to Rebbi (that is  Rebbi Yehuda Ha-Nasi, c. 135-217 CE.) two cases were sufficient, while Rabbi Shimon ben Gamliel insisted on three cases before ruling that there was a life threatening pattern.  Indeed the disease in boys must have been very perplexing, because (as you can see in the diagram above) not every boy would be affected. In fact, if the mother is a carrier and the father is not, there is only a 50% chance of a boy having hemophilia.  It is this fact that perhaps explains the dispute between Rebbi and Rabbi Shimon ben Gamliel regarding how many children need to exhibit the disease before we can assume that any future male child will also have it.  If every boy born in the family would have been a hemophiliac, Rabbi Shimon's ruling would have seemed unnecessarily cruel.  But since by chance, half of the boys born might not have hemophilia, the need to demonstrate the prevalence of the disease (in a society in which its genetic foundations were not known) seems eminently sensible.

In  Hemophilia A there are various genetic mutations that result in low levels of clotting factors. These levels may be only mildly decreased, or so low that severe life threatening hemophilia results. It is treated with transfusions of clotting factors which restore the levels to normal. Although these transfusions must be given several times a week in those with severe disease, there is hope that recombinant clotting factors can lengthen the time between the needed transfusions.

The hemophiliac as a shochet

The law discussed in Yevamot that forbids circumcision where there is a family history of hemophilia was certainly practiced. Later in Yevamot, the Mishnah records the case of a priest who was not circumcised -  because of the deaths of his brothers when they underwent the procedure. Tomorrow we read that a man who was not circumcised because he had hemophilia may serve as a shochet. The Talmud records not only the earliest known description of hemophilia, but the emphasis on the preservation of life as a normative Jewish practice. 

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Zevachim 22b ~ On the Physical and Mental Dangers of Circumcision

Milah knife..jpg

An uncircumcised Cohen may not, we are told in a Mishnah, offer sacrifices at the Temple in Jerusalem.  Just how did the Mishnah know this? In tomorrow's daf, we learn the answer:

זבחים כב,ב

 אמר רב חסדא דבר זה מתורת משה רבינו לא למדנו מדברי יחזקאל בן בוזי למדנו: כל בן נכר ערל לב וערל בשר לא יבא אל מקדשי לשרתני

Rav Chisdah says: We did not learn this matter from the Torah of Moses, our teacher; rather, we learned it from the words of the prophet Ezekiel, son of Buzi: “No stranger, uncircumcised in heart or uncircumcised in flesh, shall enter into My Sanctuary to serve Me” 

From the words "uncircumcised in flesh" the Talmud learns that a Cohen for whom circumcision would be life-threatening is forbidden to take part in sacrificial rites.  Earlier, (on page 15b) Rashi explains how circumcision might be dangerous:

 ערל. כהן שמתו אחיו מחמת מילה

Not circumcised: This means a Cohen whose brothers have died due to circumcision.

To understand the today's daf, we need to remind ourselves of the genetics of hemophilia. So let's go.

X-LINKED HEMOPHILIA A

The classic teaching about bleeding deaths and circumcision is found in Yevamot 64a.

יבמות סד, א 

תניא מלה הראשון ומת שני ומת שלישי לא תמול דברי רבי רבן שמעון בן גמליאל אומר שלישי תמול רביעי לא תמול... א"ר יוחנן מעשה בארבע אחיות בצפורי שמלה ראשונה ומת שניה ומת שלישית ומת רביעית באת לפני רבן שמעון בן גמליאל אמר לה אל תמולי

It was taught: If she circumcised her first son and he died, and her second son and he too died, she should not circumcise her third son, so taught Rebbi.  Rabbi Shimon ben Gamliel stated that she should indeed circumcise her third child, but [if he died] she must not circumcise her forth...Rabbi Yochanan said that there was once a case in Zippori in which four sisters had sons:  The first sister circumcised her son and he died, the second sister circumcised her son and he died, the third sister circumcised her son and he died, and the forth sister came to Rabbi Shimon ben Gamliel and he told her "you must not circumcise your son" (Yevamot 64:)

The Talmud here is describing a disease that is affected through the maternal line (hence the four sisters - all of whom seem to pass this disease on to their male children). The disease is X-linked Hemophilia A; the term X-linked indicates that the faulty gene is carried on the X chromosome, which is men is always inherited from the mother. Hemophilia A is an X-linked recessive genetic disease, first described by the American physician John Conrad Otto, who in 1803 described a bleeding disorder that ran in families and mostly affected the men. John Hay from Massachusetts published an account of a "remarkable hemorrhagic disposition" in the New England Journal of Medicine in 1813.

NEJM report of hemophilia 1813.png

If the mother is a carrier  - as were each of the four sisters in Zippori - then she has a one in four chance of passing on the disease to a child, and that affected child will always be a son:

In Yevamot, the rabbis argued over a technical point - that is, how many cases of bleeding are needed to establish a pattern. According to Rebbi (that is  Rebbi Yehuda Ha-Nasi, c. 135-217 CE.) two cases were sufficient, while Rabbi Shimon ben Gamliel insisted on three cases before ruling that there was a life threatening pattern.  Indeed the disease in boys must have been very perplexing, because (as you can see in the diagram above) not every boy would be affected. In fact, if the mother is a carrier and the father is not, there is only a 50% chance of a boy having hemophilia.  It is this fact that perhaps explains the dispute between Rebbi and Rabbi Shimon ben Gamliel regarding how many children need to exhibit the disease before we can assume that any future male child will also have it.  If every boy born in the family would have been a hemophiliac, Rabbi Shimon's ruling would have seemed unnecessarily cruel.  But since by chance, half of the boys born might not have hemophilia, the need to demonstrate the prevalence of the disease (in a society in which its genetic foundations were not known) seems eminently sensible.

In  Hemophilia A there are various genetic mutations that result in low levels of clotting factors. These levels may be only mildly decreased, or so low that severe life threatening hemophilia results. It is treated with transfusions of clotting factors which restore the levels to normal. Although these transfusions must be given several times a week in those with severe disease, there is hope that recombinant clotting factors can lengthen the time between the needed transfusions.

A Different explanation FROM Rabbenu Tam

In tomorrow's daf in Zevachim, we learn that there were indeed examples where what we call hemophilia A had been diagnosed, and as a result there were Cohanim who remained uncircumcised as adults. At least according to Rashi. But his grandson, Rabbenu Tam, has a different explanation. The Cohen did not have a clotting disorder. Rather, he was afraid of the pain of the procedure:

ערל. מפרש רבינו שלמה  בכל מקום שמתו אחיו מחמת מילה ור"ת מפרש דמומר לערלות וקרי ליה לבו לשמים לפי שאינו עושה אלא מדאגת צער המילה

According to Rabbenu Tam, this Cohen does not refuse circumcision as an act of religious rebellion. Rather, he refuses because of the pain involved in the procedure. Rabbenu Tam does not explain why the parents of the Cohen in question had not has him circumcised as a newborn - when he was in no position to object. But he makes a larger point: that a Jewish man who, out of fear, refuses to be circumcised - or to circumcise his newborn son - is not considered to be a religious rebel. Instead, he is called "one whose intentions are for the sake of heaven" - קרי ליה לבו לשמים.

Perhaps this expansive thinking of Rabbenu Tam might include today those who choose not to give their Jewish children a Brit Milah, because of concerns about informed consent.  Iceland recently introduced legislation to ban circumcision until a child reached the age of consent and could make an informed decision about his own genital future.  Rabbenu Tam would of course have wanted all Jewish newborn boys to have a Brit Milah and be welcomed into the Covenant of Abraham, but might he also understand those who felt differently?

[Partial repost from Repost from Yevamot 64a.]

Tagged: Hemophilia ACircumcision

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Avodah Zarah 27a ~ Circumcision and Hemophilia A

עבודה זרה כז, א

 ישראל שמתו אחיו מחמת מילה ולא מלוהו 

A Jew whose brothers died following their circumcisions, and was not circumcised.... 

fig1.jpg

In today's Daf Yomi, the Talmud considers whether a Jew who was not himself circumcised may serve as a mohel. This situation could arise when the newborn baby had older brothers who died from bleeding following their circumcisions. The young brother is then exempt from the mitzvah to be circumcised.

We first encountered this situation while studying Yevamot (64a) where cases of post-circumcision deaths are described. To understand the today's daf, we need to remind ourselves of the genetics of hemophilia.

X-linked Hemophilia A

יבמות סד, א 

תניא מלה הראשון ומת שני ומת שלישי לא תמול דברי רבי רבן שמעון בן גמליאל אומר שלישי תמול רביעי לא תמול... א"ר יוחנן מעשה בארבע אחיות בצפורי שמלה ראשונה ומת שניה ומת שלישית ומת רביעית באת לפני רבן שמעון בן גמליאל אמר לה אל תמולי

It was taught: If she circumcised her first son and he died, and her second son and he too died, she should not circumcise her third son, so taught Rebbi.  Rabbi Shimon ben Gamliel stated that she should indeed circumcise her third child, but [if he died] she must not circumcise her forth...Rabbi Yochanan said that there was once a case in Zippori in which four sisters had sons:  The first sister circumcised her son and he died, the second sister circumcised her son and he died, the third sister circumcised her son and he died, and the forth sister came to Rabbi Shimon ben Gamliel and he told her "you must not circumcise your son" (Yevamot 64:)

The Talmud here is describing a disease that is affected through the maternal line (hence the four sisters - all of whom seem to pass this disease on to their male children). The disease is X-linked Hemophilia A; the term X-linked indicates that the faulty gene is carried on the X chromosome, which is men is always inherited from the mother. Hemophilia A is an X-linked recessive genetic disease, first described by the American physician John Conrad Otto, who in 1803 described a bleeding disorder that ran in families and mostly affected the men. John Hay from Massachusetts published an account of a "remarkable hemorrhagic disposition" in the New England Journal of Medicine in 1813.

Hay, John. Account of a Remarkable Haemorrhagic Disposition, Existing in Many Individuals of the Same Family.  New England Journal of Medicine  1813:2;3;221-225.

Hay, John. Account of a Remarkable Haemorrhagic Disposition, Existing in Many Individuals of the Same Family. New England Journal of Medicine 1813:2;3;221-225.

 If the mother is a carrier  - as were each of the four sisters in Zippori - then she has a one in four chance of passing on the disease to a child, and that affected child will always be a son:

Courtesy  NHLBI

Courtesy NHLBI

In Yevamot, the rabbis argued over a technical point - that is, how many cases of bleeding are needed to establish a pattern. According to Rebbi (that is  Rebbi Yehuda Ha-Nasi, c. 135-217 CE.) two cases were sufficient, while Rabbi Shimon ben Gamliel insisted on three cases before ruling that there was a life threatening pattern.  Indeed the disease in boys must have been very perplexing, because (as you can see in the diagram above) not every boy would be affected. In fact, if the mother is a carrier and the father is not, there is only a 50% chance of a boy having hemophilia.  It is this fact that perhaps explains the dispute between Rebbi and Rabbi Shimon ben Gamliel regarding how many children need to exhibit the disease before we can assume that any future male child will also have it.  If every boy born in the family would have been a hemophiliac, Rabbi Shimon's ruling would have seemed unnecessarily cruel.  But since by chance, half of the boys born might not have hemophilia, the need to demonstrate the prevalence of the disease (in a society in which its genetic foundations were not known) seems eminently sensible.

In  Hemophilia A there are various genetic mutations that result in low levels of clotting factors. These levels may be only mildly decreased, or so low that severe life threatening hemophilia results. It is treated with transfusions of clotting factors which restore the levels to normal. Although these transfusions must be given several times a week in those with severe disease, there is hope that recombinant clotting factors can lengthen the time between the needed transfusions.

Later in Yevamot, the Mishnah records the case of a priest who was not circumcised -  because of the deaths of his brothers when they underwent the procedure. So this law was certainly practiced, and the Talmud records not only the earliest known description of hemophilia, but the emphasis on the preservation of life as a normative Jewish practice. 

רמב׳ם משנה תורה הל׳ מילה ב, א

הַכּל כְּשֵׁרִין לָמוּל. וַאֲפִלּוּ עָרֵל וְעֶבֶד וְאִשָּׁה וְקָטָן מָלִין בְּמָקוֹם שֶׁאֵין שָׁם אִישׁ

Everyone is qualified to perform the operation of circumcision. Where there is no adult circumcised male, (Israelite), it is performed by an uncircumcised Israelite, a bondman, a woman or a minor. 

[Repost from Yevamot 64a.]

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